Autoimmune Cytopenias in Chronic Lymphocytic Leukemia, Facts and Myths

被引:10
作者
Tandra, Pavankumar [1 ]
Krishnamurthy, Jairam [1 ]
Bhatt, Vijaya Raj [1 ]
Newman, Kam [2 ]
Armitage, James O. [1 ]
Akhtari, Mojtaba [1 ]
机构
[1] Univ Nebraska Med Ctr, Div Hematol & Oncol, Omaha, NE 68198 USA
[2] Cleveland Clin Fdn, Transfus Med Sect, Cleveland, OH 44195 USA
来源
MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES | 2013年 / 5卷 / 01期
关键词
D O I
10.4084/MJHID.2013.068
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
CLL has been defined as presence of more than 5000 small mature appearing monoclonal B lymphocytes with a specific immunophenotype in peripheral blood. It is a well-known fact that CLL is associated with autoimmune cytopenias. CLL cells are CD5(+) B lymphocytes, and usually are not the "guilty" cells which produce autoantibodies. T cell defect is another characteristic of CLL and the total number of T cells is increased, and there is inversion of the CD4/CD8 ratio. Autoimmune hemolytic anemia (AIHA) is the most common autoimmune complication of CLL and has been reported in 10-25% of CLL patients. However, the stage-adjusted estimated rate of AIHA in CLL is about 5%. Conversely, CLL is three times more common in patients who present with AIHA. Direct agglutinin test (DAT) is positive in 7-14% of CLL patients but AIHA may also occur in DAT negative patients. Autoimmune thrombocytopenia (AIT) is the second most common complication of CLL and has been reported in 2-3% of patients. DAT is positive in AIT but presence of antiplatelet antibodies is neither diagnostic nor reliable. Autoimmune neutropenia (AIN) and pure red cell aplasia (PRCA) are very rare complications of CLL and like other autoimmune complications of CLL may occur at any clinical stage. It is believed that most case reports of AIN and PRCA in CLL actually belong to large granular lymphocytic leukemia (LGL). Non-hematologic autoimmune complications of CLL including cold agglutinin disease (CAD), paraneoplastic pemphigus (PNP), acquired angioedema, and anti- myelin associated globulin are rare. Before starting any treatment, clinicians should distinguish between autoimmune cytopenias and massive bone marrow infiltration since autoimmune complications of CLL are not necessarily equal to advanced disease with poor prognosis. According to IWCLL guideline, steroids are the mainstay of treatment of simple autoimmunity. Intravenous immunoglobulin (IVIg), cyclosporine, and rituximab are used in complex, steroid refractory cases. Monotherapy with purine analogues and alkylating agents should be avoided as they may increase CLL associated autoimmune complications.
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页数:10
相关论文
共 89 条
[1]   Autoimmune neutropenia in adults [J].
Akhtari, Mojtaba ;
Curtis, Brian ;
Waller, Edmund K. .
AUTOIMMUNITY REVIEWS, 2009, 9 (01) :62-66
[2]  
Aleem A, 2008, ANN SAUDI MED, V28, P382
[3]   Rituximab in immune thrombocytopenia: transient responses, low rate of sustained remissions and poor response to further therapy in refractory patients [J].
Aleem, Aamer ;
Alaskar, Ahmed S. ;
Algahtani, Farja ;
Rather, Mushtaq ;
Almahayni, Muhamad Hitham ;
Al-Momen, Abdulkarim .
INTERNATIONAL JOURNAL OF HEMATOLOGY, 2010, 92 (02) :283-288
[4]   Identification of T-cell epitopes on the rhesus polypeptides in autoimmune hemolytic anemia [J].
Barker, RN ;
Hall, AM ;
Standen, GR ;
Jones, J ;
Elson, CJ .
BLOOD, 1997, 90 (07) :2701-2715
[5]  
Beardsley DS, 1998, TRANSFUS SCI, V19, P237
[6]   Reduced frequencies and suppressive function of CD4+CD25hi regulatory T cells in patients with chronic lymphocytic leukemia after therapy with fludarabine [J].
Beyer, M ;
Kochanek, M ;
Darabi, K ;
Popov, A ;
Jensen, M ;
Endl, E ;
Knolle, PA ;
Thomas, RK ;
von Bergwelt-Baildon, M ;
Bebey, S ;
Hallek, M ;
Schultze, JL .
BLOOD, 2005, 106 (06) :2018-2025
[7]  
BINET JL, 1981, CANCER-AM CANCER SOC, V48, P198, DOI 10.1002/1097-0142(19810701)48:1<198::AID-CNCR2820480131>3.0.CO
[8]  
2-V
[9]   TREATMENT OF CHRONIC LYMPHOCYTIC-LEUKEMIA [J].
BINET, JL ;
FENAUX, P ;
MOREL, P ;
BAUTERS, F ;
PIGUET, H ;
MONCONDUIT, M ;
TILLY, F ;
DESABLENS, B ;
CLAISSE, JF ;
ADJIZIAN, JC ;
POTRON, G ;
PIGNON, B ;
LEPORRIER, M ;
TROUSSARD, X ;
REMAN, O ;
BINET, JL ;
MALOUM, K ;
MERLEBERAL, H ;
RAPHAEL, M ;
REISENLEITER, M ;
LINASSIER, C ;
LAMAGNERE, JP ;
JAUBERT, J ;
VASSELOM, C ;
LEBLAY, R ;
JACOMY, D ;
GROSBOIS, B ;
DREYFUS, B ;
GUILHOT, F ;
VAUGIER, G ;
SOUTEYRAND, P ;
LEPEU, G ;
TOUCHIAS, AM ;
BOUDJERRA, N ;
BORDESSOULE, M ;
TRAVADE, P ;
SCHMIDT, J ;
FACQUETDANIS, J ;
SEBBAN, C ;
ALLARD, C ;
ROUSSET, T ;
NAVARRO, M ;
SCHVED, JF ;
ARNAUD, A ;
CASASSUS, P ;
LEPRISE, PY ;
DOR, JF ;
TURPIN, F ;
SOLALCELIGNY, P ;
TERTIAN, G .
BAILLIERES CLINICAL HAEMATOLOGY, 1993, 6 (04) :867-878
[10]   How do CD4+CD25+ regulatory T cells control autoimmunity? [J].
Bluestone, JA ;
Tang, QZ .
CURRENT OPINION IN IMMUNOLOGY, 2005, 17 (06) :638-642